Sistemik başlangıçlı juvenil idiyopatik artrit tanılı olguların değerlendirilmesi

Year 2019, Volume: 17 Issue: 2, 279 - 289, 02.08.2019

Şükrü Çekiç Yasin Karalı Sara Şebnem Kılıç

Abstract

GİRİŞ ve AMAÇ: Sistemik başlangıçlı juvenile
idiyopatik artrit (JİA) nadir görülen bir JİA alt tipi olarak kabul edilir ve
diğer JİA alt gruplarından farklı olarak otoinflamatuar hastalık grubunda
değerlendirilir. Ülkemizde sistemik başlangıçlı JİA ile ilgili az sayıda
çalışma bulunmaktadır. Bu çalışmada kliniğimizde sistemik başlangıçlı JİA
tanılı olguların klinik ve laboratuvar özelliklerinin araştırılması
amaçlanmıştır.

YÖNTEM ve GEREÇLER: Kliniğimizde takip edilen 303 JİA tanılı olgu tarandı ve 18
sistemik başlangıçlı JİA tanılı olgu (%5,9) tespit edildi. Olguların verileri
elektronik dosya kayıtlarından geriye dönük olarak taranarak elde edildi.

BULGULAR: Olguların yaşları ortanca 11,7 yıl (5,3-20 yıl) ve kız erkek oranı
4/14 idi. Tanı yaşları ortanca 4,9 yıl (1,5-15,2 yıl) ve tanıda gecikme süresi
ortanca 4 hafta (2 hafta-10,8 yıl) olarak saptandı. En sık başvuru yakınması
olguların tümünde görülen ateş ve artrit (n=18, %100) idi. Bir olguda
monoartrit görülürken diğer olgularda birden fazla eklem tutulumu vardı. En sık
görülen form monosiklik formdu (n=7, %38,8). Olgularda görülen en sık
komplikasyon boy kısalığı (n=4, %22,2) idi ve makrofaj aktivasyon sendromu 2
olguda (%11,1) gelişti. Tedavide en sık steroidler (n=17, %94,4) kullanılırken
biyolojik tedavi 7 olguda (%38,8) kullanıldı. Bu olguların son tedavilerine
bakıldığında; 5 olgunun tocilizumab, bir olgunun canakinumab aldığı, kalan 1
olgunun ise takibi bıraktığı saptandı. Takipten çıkan bir olgu dışında tüm
olgularda remisyon sağlandı.

TARTIŞMA ve SONUÇ: Sistemik başlangıçlı JİA, diğer JİA alt gruplarına kıyasla
daha az olarak görülmektedir. Hastalık enfeksiyon hastalıkları ve diğer
inflamatuar hastalıklarla karışabildiği için tanı gecikebilmektedir.
Kortikosteroid tedavisine çoğunlukla olumlu yanıt alınmakla birlikte steroide
dirençli olgularda anti IL-6 ve anti IL-1 ilaçlar etkili olabilmektedir

Keywords

Sistemik başlangıçlı juvenil idiopatik artrit, otoinflamatuar hastalık

Evaluation of patients with systemic onset juvenile idiopathic arthritis

Abstract

INTRODUCTION:
Systemic juvenile idiopathic arthritis (sJIA) is a rare, systemic inflammatory
disease classified as a subtype of JIA. There are few studies on sJIA in our
country. The aim of this study was to investigate the clinical and laboratory
features of patients with sJIA.

METHODS: Three hundred three cases with JIA followed up in our clinic were
screened, 18 of them (5.9%) diagnosed with sJIA wereincluded to our study. The
data was obtained from the electronic file records retrospectively.

RESULTS: The median age of the patients was 11.7 years (5.3-20 years), the
female/male ratio was 4/14, and the median diagnosis age was 4.9 years
(1.5-15.2 years). The median diagnosis delay was 4 weeks (2 weeks-10.8 years).
The most common complaints were fever and arthritis which were seen in all
cases (n = 18, 100%). One patient had monoarthritis while the other cases had
more than one joint involvement. The most common form was the monocyclic form
(n = 7, 38.8%). The most common complications were short stature and macrophage
activation syndrome which developed in 2 cases. Almost all patients were
treated by corticosteroids (n = 17, 94.4%), biological therapy was used in 7
patients (38.8%). Biological treatments were tocilizumab in five patients,
canakinumab in one and anakinra in 1 one patient. Remission was achieved in all
cases except one patient who was out of follow-up.

DISCUSSION AND CONCLUSION: SJIA can be challenging to diagnose. Fever and rash
in sJIA clinic may inadvertently be attributed to infectious disease.
Corticosteroids were the main treatment modalities in treatment, whereas in
resistant cases biologicals were highly effective and safe.

Keywords

Systemic onset juvenile idiopathic arthritis, autoinflammatory disease

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